CASE REPORT Disseminated linear calcinosis cutis associated with the Koebner phenomenon in an infant with congenital acute monocytic leukaemia E.K.SATTER, C.H.MAARI, K.D.MOREL, L.F.EICHENFIELD, B.B.CUNNINGHAM, S.F.FRIEDLANDER AND J.N.BERGMAN Department of Paediatric Dermatology, Children’s Hospital ⁄ University of California San Diego and Department of Dermatology, Naval Medical Center San Diego, San Diego, CA, U.S.A. Accepted for publication 1 September 2003 Summary We present a unique case of an infant with acute monocytic leukaemia who presented at birth with multiple rubbery, erythematous to violaceous subcutaneous nodules secondary to leukaemia cutis. As these infiltrates regressed with chemotherapy, numerous white to yellow linear confluent papules appeared in a scratch-like pattern. These lesions were widely disseminated but were concentrated across her face, trunk and extremities with relative sparing of the napkin area and back. We propose that these lesions represent a form of dystrophic calcinosis cutis that occurred secondary to koebnerization in an infant with congenital leukaemia cutis. Key words: calcinosis cutis, congenital leukaemia, leukaemia cutis It has been reported that 30% of all childhood malignancies are due to leukaemia; however, it rarely presents congenitally. 1 A recent review of the literature identified fewer than 200 cases of congenital leukae- mia. 2–4 In adults, leukaemia cutis occurs infrequently, typically presents late in the disease course, and portends a poor prognosis. When leukaemia presents in children, cutaneous infiltration by leukaemic cells occurs more frequently, in 10–50% of cases. 2–7 In fact, it is so common that it is now included in the differential diagnosis of a Ôblueberry muffinÕ baby in addition to congenitally acquired infections and other neonatal malignancies. 1–9 Hypercalcaemia in children with malignancy is uncommon, but has been reported to occur with leukaemia due to impaired renal excretion of calcium or due to osteolytic lesions. 10–12 If the calcium ⁄ phos- phorus product becomes persistently elevated then metastatic calcification can occur within the vascula- ture. It is rare, however, for cutaneous calcium deposits to occur via this mechanism. Case report A term Hispanic female was born by vaginal delivery to a 17-year-old G1P0. Prenatal history was significant only for elevated a-fetoprotein. At birth, the child was noted to have diffuse rubbery, red to blue–grey nodules on her face, trunk and extremities. The nodules on her helices, periocular and perioral regions distorted her facial features, giving her a dysmorphic appearance (Fig. 1A,B). Initial investigations revealed negative serology for toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus and syphilis. A peripheral white blood cell count was dramatically elevated to 130 · 10 9 L )1 , with 61% of the lymphocytes classified as blasts. A bone marrow biopsy subsequently con- firmed the diagnosis of acute monocytic leukaemia, FAB class M5, with a karyotype 46XX, t(3;22;8) p(21;q13;p11.2). A lumbar puncture revealed leukae- mic blast cells within her cerebrospinal fluid, indicative of central nervous system involvement. Correspondence: Elizabeth K.Satter. E-mail: esatter@hotmail.com; JWSteger@nmcsd.med.navy.mil The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the United States Government. British Journal of Dermatology 2004; 150: 753–756. DOI: 10.1111/j.0007-0963.2004.05895.x Ó 2004 British Association of Dermatologists 753